(Data were presented as No. (%), the measurement data were described as the mean ± standard deviation, the count data as medians and quartiles.
Abbreviations: GA, gestational age; IQR, interquartile range; SD, standard deviation; PH, pulmonary hypertension; HFOV, high-frequency oscillator ventilation;ECMO, extracorporeal membrane oxygenation.)
All cases had evidence of pulmonary hemorrhage and hyaline membrane formation with varying degrees, as well as alveolar septa, interstitial telangiectasia congestion. Abnormalities of lobation of the lung and pulmonary dysplasia were observed in three cases; Cardiac findings included PDA, PFO, increased right ventricular volume, and mild congestion and swelling of myocardial fibers microscopically; the liver herniated into the thoracic cavity was found significant impression with normal hepatic lobular structure with hepatocyte swelling and degeneration, and significant congestion microscopically; diffuse congestion and bleeding in other organs were observed. The diaphragmatic muscle tissue on the defect side was significantly reduced, hyperplastic fibrous connective tissue was more common, and the contralateral side was normal. The autopsy results showed a consistence with the clinical cause of death.
Macroscopically, the lung volume of the ipsilateral side was significantly smaller than the contralateral side (Fig 1 A), the average proportional weight of ipsilateral vs. total lung weight (%) was 25.06%±6.99%, the average LBWR was 0.0030±0.0023. The surface of the lung tissues was dark red and smooth. And the floating test in water was positive. 3 (37.5%) of them had lobulated deformity of the ipsilateral lung while bilobate, trilobate and quadrilobate lungs were found; We found 4 patients with right CDH had poor air content in both lungs. And 1 patient with left large defect had severe right curvature of the spine, involving the T5- L5 interval, showing that the left thoracic cavity was significantly enlarged compared with the right thoracic cavity. Microscopically, diffuse pulmonary hemorrhage and hemosiderin laden macrophages were observed in bilateral lung; hyaline membrane was formed in 7 cases; diffuse acute and chronic inflammatory cell infiltration was observed in alveolar space, alveolar septum was significantly widened, and capillaries were dilated and congested; exfoliated mucosal epithelial cells, inflammatory exudates and hyaline membrane were observed in small airways, which completely blocked the lumen (Fig 1 B-E); interstitial vessels were significantly dilated and congested with significant edema, pulmonary dysplasia was only found on the ipsilateral sides in 2 cases, bilaterally poor development was observed in 2 cases (bilateral congenital eventration and right CDH)(Fig 1 D-E), acinar hypoplasia was found in the pseudoglandular and canalicular stages of lung development with reduced alveoli and small lumen. 4 patients (50%) had fair lung development (1 on the left side and 3 on the right side) (Fig 1 B-C). Muscular arteries showed media thickening, sometimes with complete occlusion of the lumen (Fig 2).